subcutaneous immunoglobulin in de-novo cidp (sidec)

Conditions: CIDP - Chronic Inflammatory Demyelinating Polyneuropathy

Summary: sidec - (subcutaneous immunoglobulin in de-novo cidp) ia a study designed as a randomized, parallel study with an open-label extension phase. the aims are to compare the effect of scig and ivig in 60 treatment-naïve cidp patients, and to detect the lowest effective dosage for maintenance treatment.

in fase i the patients are followed for 26 weeks on a fixed dose of 0.54 g/kg/week in the scig group (total 14 g/kg) and 2 g/kg/4week in the ivig group (total 14 g/kg). the patients automatically continue in fase ii in which treatment is reduced every 12 weeks (90%, 75%, 50%, 25% and 0%) over a course 60 weeks. the patients are evaluated at every visit with overall disability sum score (odss), grip strength, medical research council score (mrc-score), incat-sensory sum score (iss), 10-meter-walk test (10-mwt), 6-spot-step test (6-sst), 9-hole-peg test (9-hpt), quality of life (eq-5d-5l), fatigue severity scale (fss), neuropathic pain symptom inventory (npsi), rasch built overall disability scale (rods) and life quality index (lqi) and blood samples.

Eligibility criteria:
inclusion criteria: * fulfilling efns/pns criteria for definite, probable or pure motor cidp. * no previous treatment with ivig or scig. * age ≥ 18. * odss ≥ 2 - either (arm/leg): 1/1, 2/0 or 0/2 at the time of inclusion. clinical criteria for typical cidp * chronically progressive, stepwise, or recurrent symmetric proximal and distal weakness and sensory dysfunction of all extremities, developing over at least 2 months; cranial nerves may be affected. * absent or reduced tendon reflexes in all extremities. criteria for pure motor cidp • pure motor affection; otherwise as for typical cidp. electrophysiological criteria for cidp 1. motor distal latency prolongation ≥50% above uln in two nerves (excluding median neuropathy at the wrist from carpal tunnel syndrome), or 2. reduction of motor conduction velocity ≥30% below lln in two nerves, or 3. prolongation of f-wave latency ≥30% above uln in two nerves (≥50% if amplitude of distal negative peak cmap ≤80% of lln values), or 4. absence of f-waves in two nerves of these nerves have distal negative peak cmap amplitudes ≥20% of lln + ≥1 other demyelinating parameter in ≥1 other nerve, or 5. partial motor conduction block: ≥50% amplitude reduction of the proximal negative peak cmap relative to distal, if distal negative peak cmap \>20% of lln, in two nerves, or in one nerve + ≥1 other demyelinating parameter in ≥1 other nerve, or 6. abnormal dispersion (≥30% duration increase between the proximal and distal negative peak cmap) in ≥2 nerves, or 7. distal cmap duration (interval between onset of the first negative peak an return to baseline of the last negative peak) increase in ≥1 nerve (median ≥6.6 ms, ulnar ≥6.7 ms, peroneal ≥7.6 ms, tibial ≥8.8 ms) + ≥1 other demyelinating parameter in ≥1 other nerve electrophysiological criteria for probable cidp (a) ≥30% amplitude reduction of the proximal negative peak cmap relative to distal, excluding the posterior tibial nerve, if distal negative peak cmap ≥20% of lln, in two nerves, or in one nerve + ≥1 other demyelinating parameter in ≥1 other nerve exclusion criteria: * other causes of neuropathy * increased risk of thromboembolism * pregnancy (plasma hcg is tested at inclusion in all fertile women) * breast feeding * malignancy * severe medical disease * other immune modulating treatment than low dose steroid (prednisolon \< 25 mg daily) within the last 6 months prior to inclusion * hepatitis b or c or hiv infection (screening at inclusion) * known iga deficiency * known allergy to consents in privigen or hizentra * body weight \> 120 kg after treatment initiation: * pregnancy * serious medical disease that affects treatment or examinations * non-compliance to treatment * initiation of other immune modulating therapy * unacceptable side effects * withdrawal of consent to participate (drop-out)

Keywords: immunoglobulins, intravenous injections, subcutaneous